Friday, September 4, 2015

Neurosurgery Appt, We have a plan

To continue a long week of Appts., I saw Dr.Bragg yesterday (Thurs, 3rd). The Appt went well I think it'd have to say.. these appts usually do, she is so down to earth, explains things using her funny analogies (which I fully think everyone should have to hear atleast one, they always make me smile) and she just seems to get that albeit I like her, I don't really want to be there asking for her help (if that makes sense)..
I think I wrote about this in my last post but she always asks about the heart stuff, honestly I think maybe it makes her a little nervous (which there's nothing wrong with that if it is the case), better a cautious, thoughtful Provider then 1 who thinks only of their particular treating issue OR just plain doesn't care about the whole picture!
We talked about the current shunt valve and the irritation this is causing + how the Codman Hakim valve even though it is imperfect as far as draining wise it is the best I feel like as far as shunt valves we've tried and consistent drainage vs this current valve seems very inconsistent and a lot of spine/nerve irritation.. That may not make a lot of sense but while it doesn't drain enough CSF (spinal fluid) when we've had that valve in the various shunts it seems to drain pretty constantly so atleast there isn't the nerve irritation and isn't intermittent issue..
I am unsure how exactly to describe what it is about the Codman Hakim valve that the other 3 or 4 or so valves we've tried over the past 4 yrs including the 1 I currently have in place on my shunt haven't done well.
She had looked in to see if there was a Codman valve (this is the brand, not a type of shunt, for instance Nike is a brand, 'Air Jordan' is the product.. if that makes sense?) that drains lower than the current valve I have but was unable to fine one. Not surprising I guess, and we did talk about would that have caused worse irritation to spine nerves, but is hard to know.
We've decided initially to take out this current valve given it is causing so much issue and to re-replace a Codman Hakim valve in the LP Shunt.
This will occur Sept 22nd, in the interim b/c I have a few things going on and wanted to try and postpone the surgery(Sun School starts a wk from Sun, Conf. in Utah 2wks from this past Weds) Dr.Bragg is going to do shunt taps to take off CSF next Thurs and the following Tues, before I fly out to Utah on Weds. I am beyond grateful to her she will do these taps! While the relief is short term headache wise the other symptoms seem to last longer (it is weird), a few days sometimes and it is awesome! It makes dealing with the headaches more tolerable.
I did ask her about if she was against putting in the 2nd shunt after she and I talked about the LP Shunt and changing the LP Shunt valve 1st. She isn't entirely opposed to replacing the 2nd shunt, albeit if we do that she does not want to do it at the same time as the LP Shunt valve due to infection risk and I think she wanted to give the new heart med a bit more time to have it's hopeful affect on the heart before putting my body through a tougher surgery like a VP Shunt replacement/insertion surgery.
She commented how she didn't really want to put me through 2 surgeries but overall she would feel that was safer, which I can understand. It would suck but to feel better I'd probably jump through burning hoops, so...
It seems like as I mentioned above she is willing to put the 2nd shunt back, she made a comment along the lines about giving this shunt valve change once it is done some time, maybe do the 2nd shunt later in fall? I am not entirely sure on that. Atleast she seems fairly open to the idea? She asked me what my thoughts where re where a 2nd shunt would drain ie what where my thoughts on both shunts draining to the peritoneal cavity (abdomen kind of) giving we know heart surgery is going to happen at some pt...
Basically regarding heart surgery it is a 3-6mo at a time call, I see Dr.Earing every 3mo and Echo's are done every 6mo where we re-visit a 3rd Heart surgery and 'can we postpone it some more' each time..
I see my Heart Rhythm dr in-between those visits as well and he talks with Dr.E so all are on the same page.
Given the unfortunate knowledge of heart surgery in my future and given pleural spaces (area around the lungs) are needed during heart surgeries we know it wouldn't be ideal to put a 2nd shunt if we put it back in to drain to a pleural location. That said we agreed, should we put the 2nd shunt back, both would drain to same location in the peritoneal location.
Perhaps at some pt. further down the road, after a heart surgery where over with we could move 1 of the shunt distal ends to pleural space.. While it isn't ideal having both drain to same location at this point I'd take that and probable, potential to feel some better pressure wise!
And if we're lucky this new heart med will work for a good long while and we wont have to face heart surgery for awhile..! I am hoping, I think (I know) everyone on my Team is hoping for this!

On an un-related, related note I saw my Physical Therapist today, he was impressed w how little edema (fluid?) there was today in my face and joints - joints especially and felt like he was really able to make a impact today on getting my shoulders to move better (a significant source of discomfort for me). He has also commented how in general areas like my legs, face and upper body just seem much less "puffy" (I've lost around 4pds but I don't think it is this weight loss (unintentional) that affects the less puffiness he notices. Even the heart meds (that make me pee like a sive) don't seem to touch the puffiness he has commented on from time to time.
His counterstrain/myofascial work is AMAZING though! He is like a god send when it comes to helping the muscle/nerve pain and in turn I am able to take less pain meds. We've never found a good way to effectively help the headaches but that I truly think i more b/c it is from the storage issue/MPS.
I DO think the shunt being back in LP location even if it doesn't drain enough and the new heart med even if the heart med is not yet having a significant impact on heart rate/breathing is seem to be impacting this overall puffiness. I asked Tom about it and he thought both of these (shunt and new heart med) could definitely be why he's seeing this change. Whatever the cause, if it's helping him help me i'll take the improvement!
In any case, thanks for stopping by,
Stay tuned,
Erica


Tuesday, September 1, 2015

Cardiology F/up, post Heart Studies. Neurosurgery f/up Thurs.

Follow up today with Dr.Earing (Cardiologist) - both the usual reminders of what's ahead and all that's going on (as if I can forget, living it day to day) and some funny moments, good laughs I have to share. =)
Re the Mitral valve/heart stuff, per the Heart Cath/Heart Rhythm Study back in July and the Echo back in June we know the mitral valve is in the severe range for leaking/narrowing and we know pulmonary pressures (having to do with oxygen between the heart and lungs I believe) is elevated, (which isn't a good thing) and we know there is signs of heart failure despite all the meds we've tried.
We've been on the Ivabradine for 2wks now, with heart rate today at 92, per the Fellow (a graduated MD done with her Residency training to be an Adult Congenital Heart dr.) Obviously the heart rate isn't ideal as the goal of the Ivabradine is to bring heart down to I believe below 70.
This said, studies done in Europe show it can take up to a month to show significant improvements so we're all (Dr.Kovach, Dr.Earing and myself) kind of hoping for that.. No one on my Heart Team is ready to take the significant risk of a 3rd open heart surgery yet. As Dr.Kovach says "we're pulling things out of our pocket to try and help you, to try and avoid surgery as long as possible".
Dr.Earing just said again today what Dr.K said last wk, should HR not be consistently where they'd like or symptoms not be a lot improved they'd try doubling the Ivabradine dose (10mgs 2x's/day). Given it doesn't seem to have side effects (anyone who knows/takes heart meds knows this IS kind of AMAZING!) I would be ok with that, if needed. Better than rushing to OHS!
Dr.E's exact words (or close) regarding heart surgery, which might I add, ironically it is 2yrs ago Thurs when the 2nd re-do AVR/Konno procedure was done where: "It's bad but i'm not ready to push the button yet, we just wait as long as possible and hope this med works. You know I'm dragging my feet as long as I can on doing this".
Elmiron
Separately Dr.Earing brought up about the Elmiron, the drug my Metabolic Genetics dr. at Boston Children's had called him about. Dr.E is interested in the drug and thinks we should give it a try albeit he cont's to feel now is not a good time given the many other complicating issues going on we're sorting out (heart issues, shunt issues) and worries that would make an already complicated picture even more so. I don't disagree with him and am fine waiting, I don't think right now would be a great time to start it. The Ivabradine also appears to affect the Coumadin dosing so they've adjusted that dose upwards and i'll test again next wk.
Insurance (funny stuff)
I don't quite remember how we got to talking about Insur., other than maybe Dr.E, when he came in made a comment along the lines: "I don't know how you managed to get the Ivabradine covered but I think you need to come work for me, then when my other pts. can't get their stuff covered you can just do it for them, for me". - LOL!
I know I said to him, laughing, you don't really question how I got it covered do you? =) His response, something like, no, I know you, but it's still amazing and you do a great job on this".
Sometimes my stubborn, sometimes pushy and persistent nature comes in handy BUT it is nice that he realizes it's not simple stuff to deal w the insur. stuff and appreciates what it takes as a patient to be doing what Providers normally do. I don't mind, doing this sort of thing, it makes me feel like I am helping with my care but I really appreciate that Dr.E and Jake, my Pharmacist (Skywalk) are kind enough to want my help. =)
Dr.E made the additional comment "I don't know how you do it" as he knows exactly how difficult some of my Insur. company stuff can be, he has other patients w the same/similar Dean coverage. albeit in my case I work directly with a Nurse Case Manager and have in place 'out of network approvals for a yr at a time for Echo's and Appts with him. I do not believe that is the case with other of his pts. w the same insurer.
His Fellow said something along the line after that comment by Dr.E. about "I needing to work for him", "I need to take you with me". LOL, I honestly don't think anything I do re getting Prior Auth's in place for Dr.Earing, Dr.Bragg and some of my other Providers is all that special nor do I think working on the med stuff on my own to help them is anything special but I guess it's something few pts. must do? Must be part of having an independent, I want to get this taken care of streak? In any case.. =)

And on a last note, just as Dr.Bragg always asks me about the heart stuff, Dr.Earing always asks me about the shunt stuff. Before updating him he says: 'I know you just had something done, dumb it down for me, I'm not a Neurosurgeon, I know you know more about this stuff than I do". LOL, that made me laugh! I kind of doubt that's true but non-the-less, pretty funny =)

Afterwards he said to me as we (Dr.E, Fellow and myself) where figuring out the new Coumadin dosing and f/up timing (3mo Echo/Appt unless anything changes, Dr.Kovach keeps him updated and I see him the 24th) he says regarding the shunt stuff:
"I just wish this Neurosurgery stuff where easier for you". Obviously I do to, but most of all I so appreciate he doesn't question Dr.Bragg, he gets that she's doesn't make hasty decisions. He gets that it's complicated and he appreciates she hasn't given up or abandoned my care (he saw all the issues with the 1st Nrsgn and the subsequent issues finding 1 after that who would not only follow my care but treat me and 1 who would believe me/believe those of my drs. like him who did believe I was having a shunt/pressure issue before I found Dr.Bragg).

I absolutely know he is protective and at times questions some aspects of my overall care but I am not sure he has ever had reservations about Dr.Bragg despite how complex of times we've had. I appreciate that b/c I really like him. =)

Neurosurgery F/up, Dr.Bragg, Thurs.
On a side note, I am sitting here, having taken to much zofran already, my stomach is super on edge, in all honesty I have a feeling unless I take some ativan (yuck) it is going to end up with my throwing up which super sucks. I am trying really, really hard to ignore it. Ugh, I wish my stomach would calm down!
Anyways, I see Dr.Bragg Thurs., f/up from the surgery. I am a little nervous, not b/c of actually seeing Dr.Bragg but b/c I am hopeful she will have an idea how to help when I see her for f/up. I am still hopeful she'll agree to put back the 2nd shunt but I know I have to trust or listen to whatever she might propose or if she has a different thought.
I am trying to not get my hopes up but honestly I am so, so miserable the past week-ish I just want to feel better.

I honestly think the current valve works but I don't think it works as consistently as the Codman Hakim valve did, perhaps b/c it is smaller (?) and it seems to cause extreme nerve irritation in my mid-back + my low back has literally felt like the muscles are well on fire or something (the muscles down closest to my butt actually).
It is almost funny to me, the Codman Hakim valve didn't cause the nerve irritation and did atleast drain some fluid, not enough and seemed to work consistently, just again not enough.

This valve seems to work very inconsistently and when it does seems to help headaches some but causes the nerve irritation and low back stuff and well it just is a pain overall! Ugh! Out of any shunt valve we've ever tried I'd have to say the Codman Hakim's where the best even if not the best re how much drainage. To bad they cant make them so they drain just a bit more! I honestly just have wondered if that valve where in say the LP Shunt and another fixed valve where in a VP Shunt would it be really good or a lot closer to good? I think so but that's just me..
Anyways, from time to time I am getting what I can best describe as a 'zingy' sensation along the shunt tract so I feel like a bit of a mess! The pain between my shoulder blades makes things like showering, especially washing my hair which I do every morning harken back to the 1st LP Shunt I had. I am so hopeful maybe my Physical Therapist can help with this again but I do know without a doubt some of it has to do w the valve. It started some days after the new valve was placed and I think I just didn't notice it when in-pt as much b/c of sitting so much and I didn't shower everyday there. Who knows?
In any case the week cont's to be busy, I see the Neurology doc tmrw for botox (I'd have rescheduled given this week is ridiculous with Appts but had to reschedule in July and in Aug. and I think it could possibly help the muscles to. Tmrw night we have our last planning mtng before Sunday School starts in a couple wknds and then Dr.Bragg Thurs + PT (thankfully that is local) on Fri.
Thanks for stopping by, stay tuned,
Erica
I attached some screenshots from a heart rate app I have on my phone, this is not actually a significantly high heart rate for most people, it is high but not abnormally so I guess you'd say. In the case of myself per Dr.E b/c there is already the other heart meds including/especially the Digoxin (works on heart muscle, to help the heart relax) heart rates should not be as high as they are. Even rates in the 80s are uncomfortably high to him, in my situation due to the mitral valve mixed disease (stenosis/narrowing) and the increased pulmonary pressures/Heart failure.